Lady Windermere syndrome

Abstract

Non-tuberculosis Mycobacterium spp (NTM) pulmonary disease is increasing in incidence
and is a common cause of undiagnosed lung disease in older patients. NTM pulmonary
disease occurring in patients without preexisting lung disease was only recently described by
Prince in 1988. In 1992, Reich and Johnson presented a case series of six women describing
a predilection of Mycobacterium spp pulmonary disease for the middle lobe, and its homolog,
the lingula, in elderly women without preexisting pulmonary disease. Later high resolution
computed tomography studies (HRCT) showed that the characteristic image findings in these
cases were nodules and bronchiectasis most commonly occurring in the middle lobe and
lingula. This subtype of disease is now usually referred to as nodular bronchiectasis, and
some researchers have doubted whether there really is a predilection for the middle lobe.
Although Reich and Johnson hypothesized that cough suppression in “polite” women was the
mechanism of disease, there are no large studies which support this idea. Mutations in the
cystic fibrosis transmembrane receptor, unique skeletal phenotypes, and impaired function of
the modulators of granuloma formation are the most common characteristics found in patients
with nodular bronchiectasis. These patients usually respond well to clarithromycin-based multidrug
regimens, but surgery is sometimes required to resect the infected regions of the lung.